© 2014, Turkish Ophthalmology Society. All Rights reserved.Angiomyxoma, which is a rare mesenchymal tumor, has a propensity for occurring in the trunk, head and neck, extremities, and the genital region. Development of the tumor in the orbit is extremely rare, and only a few studies have been reported to date. In this study, we present a 26-year-old female patient who was admitted with a complaint of growing mass at her left upper eyelid during the previous 9 months. Magnetic resonance imaging revealed a well-demarcated lesion appearing as hypointense on T1- and hyperintense on T2-weighted images in the left orbit; the mass was totally excised. Histopathological examination revealed a tumor comprising spindle shaped cells in myxoid stroma sprinkled with small vascular structure. The tumor tissue was positive for alcian blue and vimentin, and not for S100 on staining. Histopathological examination led to the diagnosis of orbital angiomyxoma. The case was followed-up for 18 months without any evidence of recurrence.